Diffuse cutaneous mastocytosis with novel somatic KIT mutation K509I and association with tuberous sclerosis.

Diffuse cutaneous mastocytosis (DCM) is a rare but potentially fatal condition when diagnosis and targeted treatments are delayed. This case illustrates the life-threatening complications in DCM and reviews the currently available treatments. To our knowledge, this is the first report of mastocytosis with somatic K509I mutation and concomitant tuberous sclerosis

The Trouble with Truth-telling: Preliminary Reflections on Truth and Justice in Post-war Liberia

This study investigates perceptions of the Liberian Truth and Reconciliation Commission (TRC), particularly focusing on understandings of, and the links between, truth, justice, and reconciliation. Forty-five semi-structured interviews were conducted at three research sites in Liberia. Findings indicate that although most Liberians agreed with the TRC in principle, most of those who followed its proceedings saw major problems in its implementation, harming perceptions of reconciliation. Participants expressed concerns that the Commission had failed to discover the full truth of wartime abuses, that the truth that was discovered was not told in the right way, and that there had been problems implementing justice. The data indicates that societies recovering from violence and suffering must think carefully about how to revisit their pasts. In order for a truth commission to have a positive impact, it must ensure that truth is told in a reconciliatory fashion, and that its justice-based strategy enjoys popular support

Concrete House

These concrete screens are arranged in various dynamic ways; leaning, elevated or floating and form the outer carapace to the house. Within this, courtyards are separated off by glass, which threads between the concrete screens. This brings the house in active and equivalent relationship with the landscape.https://openscholarship.wustl.edu/bcs/1175/thumbnail.jp

Mast cell-related disorders presenting with Kounis syndrome

Letter to the Editor.-- et al.Peer Reviewe

Computer integrated manufacturing (CIM) and the principle of business control

In response to the increasing international competitiveness, many manufacturing businesses are rethinking their management strategies and philosophies towards achieving a computer integrated environment. The explosive growth in Advanced Manufacturing Technology (AMI) has resulted in the formation of functional "Islands of Automation" such as Computer Aided Design (CAD), Computer Aided Manufacturing (CAM), Computer Aided Process Planning (CAPP) and Manufacturing Resources Planning (MRPII). This has resulted in an environment which has focussed areas of excellence and poor overall efficiency, co-ordination and control. The main role of Computer Integrated Manufacturing (CIM) is to integrate these islands of automation and develop a totally integrated and controlled environment. However, the various perceptions of CIM, although developing, remain focussed on a very narrow integration scope and have consequently resulted in mere linked islands of automation with little improvement in overall co-ordination and control. This thesis, that is the research described within, develops and examines a more holistic view of CIM, which is based on the integration of various business elements. One particular business element, namely control, has been shown to have a multi-facetted and underpinning relationship with the CIM philosophy. This relationship impacts various CIM system design aspects including the CIM business analysis and modelling technique, the specification of systems integration requirements, the CIM system architectural form and the degree of business redesign. The research findings show that fundamental changes to CIM system design are required; these are incorporated in a generic CIM design methodology. The affect and influence of this holistic view of CIM on a manufacturing business has been evaluated through various industrial case study applications. Based on the evidence obtained, it has been concluded that this holistic, control based approach to CIM can provide a greatly improved means of achieving a totally integrated and controlled business environment. This generic CIM methodology will therefore make a significant contribution to the planning, modelling, design and development of future CIM systems

Ex vivo identification and characterization of a population of CD13high CD105+ CD45− mesenchymal stem cells in human bone marrow

This article is distributed under the terms of the Creative Commons Attribution 4.0 International License.[Introduction]: Mesenchymal stem cells (MSCs) are multipotent cells capable of self-renewal and multilineage differentiation. Their multipotential capacity and immunomodulatory properties have led to an increasing interest in their biological properties and therapeutic applications. Currently, the definition of MSCs relies on a combination of phenotypic, morphological and functional characteristics which are typically evaluated upon in vitro expansion, a process that may ultimately lead to modulation of the immunophenotypic, functional and/or genetic features of these cells. Therefore, at present there is great interest in providing markers and phenotypes for direct in vivo and ex vivo identification and isolation of MSCs. [Methods]: Multiparameter flow cytometry immunophenotypic studies were performed on 65 bone marrow (BM) samples for characterization of CD13high CD105+ CD45– cells. Isolation and expansion of these cells was performed in a subset of samples in parallel to the expansion of MSCs from mononuclear cells following currently established procedures. The protein expression profile of these cells was further assessed on (paired) primary and in vitro expanded BM MSCs, and their adipogenic, chondrogenic and osteogenic differentiation potential was also determined. [Results]: Our results show that the CD13high CD105+ CD45− immunophenotype defines a minor subset of cells that are systematically present ex vivo in normal/reactive BM (n = 65) and that display immunophenotypic features, plastic adherence ability, and osteogenic, adipogenic and chondrogenic differentiation capacities fully compatible with those of MSCs. In addition, we also show that in vitro expansion of these cells modulates their immunophenotypic characteristics, including changes in the expression of markers currently used for the definition of MSCs, such as CD105, CD146 and HLA-DR. [Conclusions]: BM MSCs can be identified ex vivo in normal/reactive BM, based on a robust CD13high CD105+ and CD45− immunophenotypic profile. Furthermore, in vitro expansion of these cells is associated with significant changes in the immunophenotypic profile of MSCs.This work was supported by grants from the Instituto de Salud Carlos III, FEDER, Ministry of Economy and Competitivity, Madrid, Spain (grant PI11/02399; RETICEF RD12/0043/0021; RTICC RD12/0036/0048); Fundación Ramon Areces, Madrid, Spain (grant CIVP16A1806); Fundación Científica de la Asociación Española Contra el Cáncer (AECC); Consejería de Sanidad, Gerencia Regional de Salud de Castilla y León (SACYL) (grant BIO/SA24/13) and Fundación Samuel Solórzano Barruso (University of Salamanca, Spain); CT was supported by a grant co-financed by the European Social Fund and the Junta de Castilla y León (Spain).Peer Reviewe

Definitions, Criteria and Global Classification of Mast Cell Disorders with Special Reference to Mast Cell Activation Syndromes: A Consensus Proposal

Activation of tissue mast cells (MCs) and their abnormal growth and accumulation in various organs are typically found in primary MC disorders also referred to as mastocytosis. However, increasing numbers of patients are now being informed that their clinical findings are due to MC activation (MCA) that is neither associated with mastocytosis nor with a defined allergic or inflammatory reaction. In other patients with MCA, MCs appear to be clonal cells, but criteria for diagnosing mastocytosis are not met. A working conference was organized in 2010 with the aim to define criteria for diagnosing MCA and related disorders, and to propose a global unifying classification of all MC disorders and pathologic MC reactions. This classification includes three types of `MCA syndromes' (MCASs), namely primary MCAS, secondary MCAS and idiopathic MCAS. MCA is now defined by robust and generally applicable criteria, including (1) typical clinical symptoms, (2) a substantial transient increase in serum total tryptase level or an increase in other MC-derived mediators, such as histamine or prostaglandin D 2, or their urinary metabolites, and (3) a response of clinical symptoms to agents that attenuate the production or activities of MC mediators. These criteria should assist in the identification and diagnosis of patients with MCAS, and in avoiding misdiagnoses or overinterpretation of clinical symptoms in daily practice. Moreover, the MCAS concept should stimulate research in order to identify and exploit new molecular mechanisms and therapeutic targets. Copyright (C) 2011 S. Karger AG, Base

Influencia de las redes sociales sobre los Trastornos de la Conducta Alimentaria: una revisión sistemática

39 p.El uso de redes sociales está cada vez más extendido entre la población general y se plantea como un factor de riesgo para el desarrollo de Trastornos de la Conducta Alimentaria. Este estudio pretende revisar la literatura científica disponible sobre el impacto de las redes sociales sobre las variables de imagen corporal y los Trastornos de la Conducta Alimentaria. Para ello se realizó una revisión sistemática de literatura científica publicada entre enero y febrero de 2024 en las bases de datos de PubMed, Scopus, Web of Science, Cohrane Library. Un total de 26 artículos fueron identificados y seleccionados. La evidencia apunta a que el uso de redes sociales está vinculado con alteraciones de la imagen corporal mediante mayor internalización del ideal de delgadez y musculatura, comparaciones físicas ascendentes más frecuentes, y con el desarrollo y/o mantenimiento de los TCA.Social media use has become increasingly prevalent among the general population and has emerged as a potential risk factor for the development of Eating Disorders (EDs). This study aims to review the available scientific literature on the impact of social media on body image variables and Eating Disorders. To achieve this objective, a systematic review of scientific literature published between January and February 2024 was conducted in PubMed, Scopus, Web of Science, and Cochrane Library databases. A total of 26 articles were identified and selected. The evidence suggests that social media use is linked to body image disturbances through increased internalization of the thinness and muscularity ideal, more frequent upward physical comparisons, and the development and/or maintenance of EDs.Grado en Medicin